The harbinger of death doesn’t always strike swiftly. Sometimes, it stalks its prey silently, hidden in the shadows for decades. This week, scientists shed light on a haunting case—a 58-year-old woman whose demise stemmed from a prion affliction contracted approximately 50 years prior.
Her story unfolds within the pages of Emerging Infectious Diseases, where medical professionals detailed her ordeal. The woman had unknowingly harbored Creutzfeldt-Jakob disease (CJD), most plausibly traced to human growth hormone (HGH) treatments she received as a child, therapies tainted by contaminated cadaveric matter. This may be the most prolonged latency ever recorded for this inevitably fatal neurological blight.
Prions, the culprits behind such disease, are aberrant versions of a naturally occurring yet enigmatic protein housed in human tissue. These deranged proteins coerce their healthy counterparts into identical malformations—reminiscent of a biological uprising, one that mirrors a sinister, zombie-like contagion, according to gizmodo.com.
As these deviant proteins proliferate, they ravage cerebral tissue, resulting in rapidly advancing dementia and death. What makes prions particularly alarming is their imperviousness to conventional sterilization techniques that easily vanquish viruses and bacteria. Fortunately, genuine prion disorders are exceptionally rare, though kindred conditions—like Alzheimer’s—afflict many more.
Prion disorders manifest in several unsettling forms. They may arise randomly, through inherited mutations, or—most disturbingly—via direct exposure to infected neural or bodily material. In the 1980s, it was discovered that individuals treated with HGH derived from infected cadavers were at risk of contracting CJD. A grim echo of this emerged again when the so-called mad cow disease outbreak revealed that prions could also cross the species boundary via tainted beef.
The revelation of CJD’s link to cadaver-sourced HGH led to an immediate cessation of such treatments in the US and other nations. Synthetic HGH thankfully supplanted it. Still, over 200 incidents of iatrogenic CJD—illnesses induced by medical interventions—have since been catalogued worldwide. While most symptoms appeared within a decade of exposure, a minority of infections hibernated quietly, biding their time.
In this woman’s case, her first symptoms appeared as unsteady limbs and trembling hands. Her condition spiraled quickly. Within a month, she was hospitalized. Days later, she fell into a coma—her last descent—never to reawaken.
Post-mortem analyses revealed hallmark brain deterioration from CJD, yet excluded inherited origins. Given the timeline and the fact that her HGH treatments predated the 1977 reform in hormone sourcing, physicians deduced her affliction stemmed from those early procedures.
Children undergoing HGH therapy typically receive it over multiple years. In this instance, her first injection occurred 51.3 years before symptoms emerged; her last was 42.1 years earlier. The US’s shift in HGH extraction methods in 1977 inadvertently reduced the prion transmission threat. Based on her treatment window, her infection most likely occurred 48.3 years prior to her death, as per gizmodo.com.
While CJD cases with latency surpassing 30 years are known, and some rare prion conditions even longer, this instance marks the longest documented delay in the onset of HGH-related CJD.
Genetic nuances play a role, too. The woman carried a specific mutation tied to delayed symptom emergence. Although cadaver-derived HGH has long been off the shelves, a spectral risk lingers. It’s believed that around 7,700 Americans received such treatment before the practice ceased—some, perhaps, unwitting carriers of this silent affliction.
Clinicians, especially neurologists, are urged to keep historical HGH treatment in mind when diagnosing unexplained cerebral declines.
As the researchers somberly noted, “Though the American iCJD wave has greatly waned, its shadow still stretches forward. New cases may yet arise.”